Inflammatory bowel disease in chronic granulomatous disease: A single center experience

2015 ◽  
Vol 47 ◽  
pp. e261
Author(s):  
G. Angelino ◽  
F. Rea ◽  
A. Claps ◽  
E. Romeo ◽  
S. Faraci ◽  
...  
Keyword(s):  
Inflammatory Bowel Disease ◽  
Chronic Granulomatous Disease ◽  
Bowel Disease ◽  
Granulomatous Disease ◽  
Single Center ◽  
Single Center Experience ◽  
Inflammatory Bowel

USTEKINUMAB FOR CHRONIC GRANULOMATOUS DISEASE-ASSOCIATED INFLAMMATORY BOWEL DISEASE

2021 ◽  
Vol 27 (Supplement_1) ◽  
pp. S59-S59
Author(s):  
Sumona Bhattacharya ◽  
Beatriz Marciano ◽  
Harry Malech ◽  
Steven Holland ◽  
Suk See De Ravin ◽  
...  
Keyword(s):  
Inflammatory Bowel Disease ◽  
Chronic Granulomatous Disease ◽  
Bowel Disease ◽  
Clinical Remission ◽  
Colonic Disease ◽  
Cell Transplant ◽  
Granulomatous Disease ◽  
Efficacy And Safety ◽  
Serious Infections ◽  
Inflammatory Bowel

Abstract Introduction Chronic granulomatous disease (CGD) is a rare immunodeficiency caused by mutations in the NADPH oxidase complex. Dysregulated immune function may cause inflammatory bowel disease (IBD). Patients with CGD-associated IBD may not respond to or may develop serious infections as a result of traditional IBD therapies such as vedolizumab and infliximab. Ustekinumab is approved for use in Crohn’s disease and ulcerative colitis however there is scarce data on its efficacy and safety in CGD. Aims To evaluate the efficacy and safety of ustekinumab for CGD-associated IBD. Methods A retrospective chart review was conducted on CGD patients followed at a single center who had consented to participate in a natural history study. Clinical, laboratory, and endoscopic data were extracted in those that had received ustekinumab for IBD. Results Eight patients were found. Four were male and four were female. Five were white, one was Asian, one was black, and one was mixed race. Median age at diagnosis of CGD was 3 years (IQR 8) and of IBD was 15.5 years (IQR 20). Median age at initiation of ustekinumab was 27.5 years (IQR 14) and median duration on ustekinumab was 10 months (IQR 7). Six had colonic disease, two had ileocolonic disease, and six had perianal disease. Six failed other biologics (n=5 for vedolizumab, n=1 for infliximab, n=1 for adalimumab). Six patients symptomatically improved whereas two had no improvement. Changes in hemoglobin and C-reactive protein were equivocal. Three patients had improved endoscopic findings, two had unimproved findings, and three patients lacked this data. Overall, four patients achieved clinical remission. However, none of the five patients with endoscopic reevaluation achieved endoscopic remission. Three patients discontinued therapy due to lack of response: two required surgery and one underwent stem cell transplant. Fungal pneumonia (n=2), otitis media (n=1), oral herpes simplex virus 1 (n=1), and viral gastroenteritis (n=1) were reported. One infusion reaction occurred. Discussion In our cohort of eight patients with CGD-associated IBD receiving ustekinumab, results were mixed with four patients experiencing some degree of clinical or endoscopic improvement including four who achieved clinical remission. Multiple CGD-related variables may account for the mixed laboratory findings. Four of the five patients with endoscopic reevaluation had pre-existing strictures that would be unlikely to reverse with medical therapy alone. Of these, two had otherwise resolved endoscopic inflammation. Only two patients had no endoscopic improvement. Two serious infections occurred however CGD confers increased infectious susceptibility and no infections lead to discontinuation of therapy. Given these promising results, further formalized study of ustekinumab in CGD-associated IBD is needed.

scholarly journals Recombinant human erythropoietin in patients with inflammatory bowel disease and refractory anemia: A 15-year single center experience

2012 ◽  
Vol 6 (1) ◽  
pp. 56-61 ◽  
Author(s):  
Konstantinos H. Katsanos ◽  
Athina Tatsioni ◽  
Dimitra Natsi ◽  
Dimitrios Sigounas ◽  
Dimitrios K. Christodoulou ◽  
...  
Keyword(s):  
Inflammatory Bowel Disease ◽  
Bowel Disease ◽  
Recombinant Human Erythropoietin ◽  
Refractory Anemia ◽  
Single Center ◽  
Human Erythropoietin ◽  
Single Center Experience ◽  
Inflammatory Bowel

Utility of Screening for Chronic Granulomatous Disease in Patients with Inflammatory Bowel Disease

2011 ◽  
Vol 32 (1) ◽  
pp. 78-81 ◽  
Author(s):  
Preeti Jaggi ◽  
Rebecca Scherzer ◽  
Rose Knieper ◽  
Hayat Mousa ◽  
Vinay Prasad
Keyword(s):  
Inflammatory Bowel Disease ◽  
Chronic Granulomatous Disease ◽  
Bowel Disease ◽  
Granulomatous Disease ◽  
Inflammatory Bowel

scholarly journals A Rare Form of Chronic Granulomatous Disease (Type Iva) Presenting as Inflammatory Bowel Disease

1996 ◽  
Vol 10 (4) ◽  
pp. 221-224 ◽  
Author(s):  
Francisco A Sylvester
Keyword(s):  
Inflammatory Bowel Disease ◽  
Chronic Granulomatous Disease ◽  
Bowel Disease ◽  
Intestinal Inflammation ◽  
Gastrointestinal Symptoms ◽  
Hypochromic Anemia ◽  
Granulomatous Disease ◽  
Poor Growth ◽  
Inflammatory Bowel ◽  
Chronic Intestinal Inflammation

Neutrophil dysfunction syndromes can sometimes mimic the clinical and pathological features of inflammatory bowel disease. The case of a 3.5-year-old boy with chronic diarrhea, abdominal pain, poor growth since infancy and microcytic, hypochromic anemia is presented. After an extensive diagnostic evaluation, he was found to have a rare variant (type IVA) of chronic granulomatous disease. His gastrointestinal symptoms markedly improved during therapy with gamma-interferon. Chronic granulomatous disease can present initially with a clinical picture suggestive of chronic intestinal inflammation. Therefore it should be considered in the differential diagnosis of atypical inflammatory bowel disease, both in children and young adults.

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